Combined assessment of sex- and mutation-specific information for risk stratification in type 1 long QT syndrome.

BACKGROUND: Men and women with type 1 long QT syndrome (LQT1) exhibit time-dependent differences in the risk for cardiac events. OBJECTIVE: We hypothesized that sex-specific risk for LQT1 is related to the location and function of the disease-causing mutation in the KCNQ1 gene. METHODS: The risk for life-threatening cardiac events (comprising aborted cardiac arrest [ACA] or sudden cardiac death [SCD]) from birth through age 40 years was assessed among 1051 individuals with LQT1 (450 men and 601 women) by the location and function of the LQT1-causing mutation (prespecified as mutations in the intracellular domains linking the membrane-spanning segments [ie, S2-S3 and S4-S5 cytoplasmic loops] involved in adrenergic channel regulation vs other mutations). RESULTS: Multivariate analysis showed that during childhood (age group: 0-13 years) men had >2-fold (P < .003) increased risk for ACA/SCD than did women, whereas after the onset of adolescence the risk for ACA/SCD was similar between men and women (hazard ratio = 0.89 [P = .64]). The presence of cytoplasmic-loop mutations was associated with a 2.7-fold (P < .001) increased risk for ACA/SCD among women, but it did not affect the risk among men (hazard ratio 1.37; P = .26). Time-dependent syncope was associated with a more pronounced risk-increase among men than among women (hazard ratio 4.73 [P < .001] and 2.43 [P = .02], respectively), whereas a prolonged corrected QT interval (≥ 500 ms) was associated with a higher risk among women than among men. CONCLUSION: Our findings suggest that the combined assessment of clinical and mutation location/functional data can be used to identify sex-specific risk factors for life-threatening events for patients with LQT1.

The future of fellowship training in clinical cardiac electrophysiology: Program directors' perspective 2008.

BACKGROUND: At the Clinical Cardiac Electrophysiology (CCEP) program directors' annual meeting during the 2008 scientific sessions of the Heart Rhythm Society, a consensus emerged for an urgent need to strengthen and rejuvenate fellowship training in clinical cardiac electrophysiology. OBJECTIVE: A writing group of the Heart Rhythm Society Clinical Research and Training Committee was charged with defining these issues. METHODS: A comprehensive questionnaire designed by the writing group was used to conduct an on-line survey of the 101 CCEP program directors in the United States. Data collected included types of programs, current status of programs, duration of fellowship, teaching responsibilities of faculty, responsibilities of fellows, and volume of electrophysiology procedures. Survey responses were collated and analyzed by the writing group. RESULTS: Given the rapid evolution and increased complexity of current electrophysiology procedures, program directors were of the opinion that 1 year of clinical electrophysiology training may no longer be adequate. A need to strengthen both research and didactic training components of fellowship training was also acknowledged. The number of electrophysiology procedures performed by trainees varied greatly between programs, and standardization of didactic training and procedural volume would be welcomed. Recent trends were recognized that indicate the need for a detailed national work-force analysis in CCEP. CONCLUSION: Through this national survey, program directors identified specific areas of need for standardization and strengthening of current fellowship training in CCEP. Based on these, specific measures can be taken to ensure the future of CCEP training.